You have been logged out of VisualDx or your session has expired.

Please reload this page and sign into VisualDx to continue.

  VisualDx Mobile   Select Language

Get VisualDx Mobile

There are VisualDx mobile apps available for iOS and Android devices.

You will need a VisualDx account to use the mobile apps.

Already have an account? Sign In or
sign up for a free trial.

Users with VisualDx accounts earn CME credits for using VisualDx.

Already have an account? Sign In or
sign up for a free trial.

Create a Personal Account

E-mail (username)
Verify Password
First Name
Last Name

Personal Account Created

Mobile Access

You can now download VisualDx for your iOS and Android devices. Launch the VisualDx app from your device and sign in using your VisualDx personal account username and password.

CME Certification

Sign in with your personal account to earn and claim CME credits through VisualDx. Credits can be earned by building a differential or looking up a diagnosis.

Version: 7.13.1441   (build df7aed4)
Select Language

Select Region

Send us your feedback

This field is required

Oops! There was an issue during submission. Please try again. If the problem persists, email with your feedback.

Thank You!

We appreciate your feedback and you will be hearing from us soon.


Share This Page

Thank You!

We have sent an e-mail with a link to the current page.


E-mail This Patient Information Sheet

Thank You!

We have sent an e-mail with this patient information.


Image Contributors


  • Christine Ahn MD
    Carl Allen DDS, MSD
    Brandon Ayres MD
    Howard P. Baden MD
    Robert Baran MD
    Keira Barr MD
    Gregory J. Basura MD, Ph.D
    Donald Belsito MD
    Jeffrey D. Bernhard MD
    Jesse Berry MD
    Victor Blanco MD
    Benjamin R. Bohaty MD
    William Bonnez MD
    Sarah Brenner MD
    Robert A. Briggaman MD
    Robert Brodell MD
    Roman Bronfenbrener MD
    Walter Brooks MD
    William Buckley MD
    Philip Bulterys MD, PhD (candidate)
    Susan Burgin MD
    Sonya Burton MD
    Sean P. Bush MD, FACEP
    Jeffrey Callen MD
    Scott Camazine MD
    Michael Cardwell
    Shelley D. Cathcart MD
    Robert Chalmers MD, MRCP, FRCP
    Chia-Yu Chu MD, PhD
    Flavio Ciferri MD
    Maria Rosa Cordisco MD
    Noah Craft MD, PhD
    John T. Crissey MD
    Harold E. Cross MD, PhD
    Charles E. Crutchfield III MD
    Adriana Cruz MD
    Donna Culton MD, PhD
    Bart J. Currie MBBS, FRACP, DTM&H
    Chicky Dadlani MD
    Alexander Dane DO
    C. Ralph Daniel III MD
    Thomas Darling MD, PhD
    William Delaney MD
    Damian P. DiCostanzo MD
    Ncoza Dlova MD
    James Earls MD
    Libby Edwards MD
    Melissa K. Egge MD
    Charles N. Ellis MD
    Rachel Ellis MD
    David Elpern MD
    Nancy Esterly MD
    Stephen Estes MD
    E. Dale Everett MD
    Janet Fairley MD
    David Feingold MD
    Jennifer J. Findeis-Hosey MD
    Benjamin Fisher MD
    Henry Foong MBBS, FRCP
    David Foster MD, MPH
    Brian D. Foy PhD
    Michael Franzblau MD
    Vincent Fulginiti MD
    Sunir J. Garg MD, FACS
    Kevin J. Geary MD
    Lowell Goldsmith MD, MPH
    Sethuraman Gomathy MD
    Bernardo Gontijo MD, PhD
    Kenneth Greer MD
    Kenneth G. Gross MD
    Alan Gruber MD
    Nathan D. Gundacker MD
    Akshya Gupta MD
    Vidal Haddad MSC, PhD, MD
    Edward Halperin MD, MA
    Ronald Hansen MD
    John Harvey
    Rizwan Hassan MD
    Michael Hawke MD
    Jason E. Hawkes MD
    Peter W. Heald MD
    David G. Hicks MD
    Sarah Hocker DO
    Ryan J. Hoefen MD, PhD
    Li-Yang Hsu MD
    William Huang MD
    Sanjana Iyengar MD
    Alvin H. Jacobs MD
    Saagar Jadeja MD
    Shahbaz A. Janjua MD
    Joshua J. Jarvis MD
    Kit Johnson
    Zachary John Jones MD
    Robert Kalb MD
    A. Paul Kelly MD
    Henry Kempe MD
    Loren Ketai MD
    Sidney Klaus MD
    Ashwin Kosambia MD
    Jessica A. Kozel MD
    Carl Krucke
    Mario E. Lacouture MD
    Joseph Lam MD
    Alfred T. Lane MD
    Edith Lederman MD
    Nahyoung Grace Lee MD
    Pedro Legua MD, PhD
    Robert Levin MD
    Bethany Lewis MD
    Sue Lewis-Jones FRCP, FRCPCH
    Taisheng Li MD
    Christine Liang MD
    Shari Lipner MD, PhD
    Adam Lipworth MD
    Jason Maguire MD
    Mark Malek MD, MPH
    Jere Mammino DO
    Ricardo Mandojana MD
    Lynne Margesson MD
    Thomas J. Marrie MD
    Maydel Martinez MD
    Ralph Massey MD
    Patrick McCleskey MD
    Karen McKoy MD
    Thomas McMeekin MD
    Josette McMichael MD
    Somchai Meesiri MD
    Joseph F. Merola MD
    Mary Gail Mercurio MD
    Anis Miladi MD
    Larry E. Millikan MD
    Dan Milner Jr. MD
    Zaw Min MD
    Stephanie Montero
    Alastair Moore MD
    Keith Morley MD
    Dean Morrell MD
    Samuel Moschella MD
    Rehan Naseemuddin MD
    Taimor Nawaz MD
    Vic Newcomer MD
    John Nguyen MD
    Matilda Nicholas MD
    Thomas P. Nigra MD
    Steven Oberlender MD, PhD
    Maria Teresa Ochoa MD
    Art Papier MD
    Lawrence Parish MD
    Tanner Parrent MD
    Mukesh Patel MD
    Lauren Patty-Daskivich MD
    David Peng MD, MPH
    Robert Penne MD
    Nitipong Permpalung MD
    Miriam Pomeranz MD
    Doug Powell MD
    Harold S. Rabinovitz MD
    Christopher J. Rapuano MD
    Sireesha Reddy MD
    Angela Restrepo MD, PhD
    Bertrand Richert MD, PhD
    J. Martin Rodriguez, MD, FACP
    Theodore Rosen MD
    Misha Rosenbach MD
    Scott Schiffman MD
    Robert H. Schosser MD
    Glynis A. Scott MD
    Carlos Seas MD, MSc
    Deniz Seçkin MD
    Daniel Sexton MD
    Paul K. Shitabata MD
    Tor Shwayder MD, FAAP, FAAD
    Elaine Siegfried MD
    Gene Sienkiewicz MD
    Christye Sisson
    Philip I. Song MD
    Mary J. Spencer MD, FAAP
    Lawrence B. Stack MD
    Sarah Stein MD
    William Van Stoecker MD
    Frances J. Storrs MD
    Erik J. Stratman MD
    Lindsay C. Strowd MD
    Erika Summers MD
    Belinda Tan MD, PhD
    Robert Tomsick MD
    Hensin Tsao MD, PhD
    Richard P. Usatine MD
    Jenny Valverde MD
    Vishalakshi Viswanath MD
    Susan Voci MD
    Lisa Wallin ANP, FCCWS
    Douglas Walsh MD
    Ryan R. Walsh MD
    George Watt MD
    Clayton E. Wheeler MD
    Sally-Ann Whelan MS, NP, CWOCN
    Jan Willems MD, PhD
    James Henry Willig MD, MPH
    Karen Wiss MD
    Vivian Wong MD, PhD
    Sook-Bin Woo MS, DMD, MMSc
    Jamie Woodcock MD
    Stephen J. Xenias MD
    Nathaniel Yohannes
    Lisa Zaba MD
    Vijay Zawar MD
    Bonnnie Zhang MD
    Carolyn Ziemer MD
    Jeffrey P. Zwerner MD, PhD


  • Am. Journal of Trop. Med & Hygiene
  • Armed Forces Pest Management Board
  • Blackwell Publishing
  • Bugwood Network
  • Centers For Disease Control and Prevention
  • Centro Internacional de Entrenamiento e Investigaciones Mèdicas (CIDEIM)
  • Dermatology Online Journal
  • East Carolina University (ECU), Division of Dermatology
  • International Atomic Energy Agency
  • Massachusetts Medical Society
  • Oxford University Press
  • Radiological Society of North America
  • Washington Hospital Center
  • Wikipedia
  • World Health Organization
ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferencesView all Images (5)
Takayasu arteritis - Skin
Other Resources UpToDate PubMed

Takayasu arteritis - Skin

Print Images (5)
Contributors: Michael Horwich MD, PhD, Nikki Levin MD, Jeffrey D. Bernhard MD, Sarah Stein MD, Karen Wiss MD, Sheila Galbraith MD, Craig N. Burkhart MD, Dean Morrell MD, Lynn Garfunkel MD, Nancy Esterly MD
Other Resources UpToDate PubMed


Takayasu arteritis, also referred to as pulseless disease and aortic arch syndrome, is a rare chronic inflammatory vasculitis that primarily affects large- and medium-sized vessels. Cutaneous involvement can occur when inflammation of the cutaneous vasculature occurs and may present as erythema multiforme, erythema nodosum, erythema induratum, pyoderma gangrenosum, ulcerated subacute nodular lesions, papulonecrotic eruptions, and papular erythematous lesions of the hands.

No definitive cause of Takayasu arteritis has been identified, but it is thought to be an IgG-mediated autoimmune vasculitis, perhaps triggered by a cross-reacting infectious agent.

The disease typically presents in the second and third decade of life in females (10:1) of Asian descent. It is most prevalent in Japan, Southeast Asia, India, and Mexico but has been found worldwide and in both sexes. Incidence is ~2.5 per million.

The disease has two phases that may overlap: a "pre-pulseless" and a pulseless phase. In the first phase, a constellation of nonspecific constitutional symptoms and signs including fever, night sweats, malaise, weight loss, arthralgia, myalgia, and mild anemia may be seen. In the second phase, the characteristic sequelae of large-vessel stenosis – upper extremity claudication, diminished brachial pulses, and/or differences in blood pressure between contralateral or ipsilateral extremities – occur.

For diagnosis, the American College of Rheumatology requires 3 of 6 criteria:
  • Age at disease onset ≤ 40 years
  • Claudication of extremities
  • Decreased brachial artery pulse
  • Blood pressure difference of > 10 mmHg between arms
  • Bruit over subclavian artery or aorta
  • Arteriogram abnormality
Although subclavian vessels are most commonly affected, other branches may be involved: carotid stenosis may cause headache, dizziness, amaurosis, or syncope; renal artery involvement frequently causes hypertension; and proximal dilatation of the aorta can cause aortic regurgitation, dilated cardiomyopathy, and congestive heart failure. Additional associated symptoms may include Raynaud's phenomenon, dyspnea, chest pain, and myocardial ischemia.

Diagnosis during the pre-pulseless phase is difficult because of the nonspecific nature of symptoms and lab abnormalities. Diagnosis is usually made during the pulseless phase when clinical criteria, as per above, are met. Angiography is essential to confirm diagnosis.

Constitutional symptoms are typically intermittent, and vascular complications are generally progressive, but the prognosis is generally good, with 5-year survival reports of 90% to 94%.

Pediatric Patient Considerations:
Takayasu arteritis in children rarely presents with pulselessness, claudication, or bruits. It is most frequently identified during evaluation for hypertension, heart failure, and neurological symptoms.

For more information, see OMIM.


M31.4 – Aortic arch syndrome [Takayasu]

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

  • Mycotic aneurism – rule out sepsis or endocarditis with blood culture
  • Tuberculosis – check PPD status
  • Tertiary syphilis – check for fluorescent treponemal antibody (~ 1/4 of RPR is false negative in tertiary syphilis)
  • Leprosy – look for hypopigmented or erythematous macules with loss of sensation, thickened peripheral nerves, and acid-fast bacilli on skin smear or biopsy
Congenital / genetic:
  • Congenital malformation – aortic coarctation or middle aortic syndrome; unlikely to have constitutional symptoms
  • Marfan syndrome – look for arachnodactyly, pectus excavatum or carinatum, and arm span greater than height; family history
  • Neurofibromatosis – look for neurofibromas, café au lait spots, ocular Lisch nodules; family history (autosomal dominant) 
  • Ehlers-Danlos syndrome – look for fragile skin, easy bruising, joint hyperextendability, frequent dislocations
  • Fibromuscular dysplasia – look for "string of beads" with angiography; family history (autosomal dominant)
  • Post-radiation therapy – may cause large-vessel stenosis
On average it takes 44 months from onset of symptoms to diagnose Takayasu arteritis. Because of its rarity and the often subtle physical findings, Takayasu arteritis frequently fails to enter the differential diagnosis for patients with fever of unknown origin (FUO). Thus it is critical to consider Takayasu arteritis in patients under 40 with FUO, aortic regurgitation, hypertension, or absent pulses. Rarely, patients older than 40 years of age meet the criteria for Takayasu arteritis because of a prolonged pre-diagnostic period or late onset of symptoms. In this case, giant cell arteritis may be indistinguishable from Takayasu arteritis; however, initial treatment for both is nearly identical. 

Best Tests

Subscription Required

Management Pearls

Subscription Required


Subscription Required


Subscription Required

Last Updated: 03/29/2017
Copyright © 2018 VisualDx®. All rights reserved.
Takayasu arteritis - Skin
Print 5 Images
View all Images (5)
(with subscription)
Takayasu arteritis (Prepulseless Phase) : Fatigue, Fever, Night sweats, Malaise, Weight loss, Arthralgia, Anemia
Clinical image of Takayasu arteritis
Copyright © 2018 VisualDx®. All rights reserved.